Background

Tricuspid valve dysfunction can result from morphological alterations in the valve or from functional aberrations of the myocardium. Tricuspid stenosis is almost always rheumatic in origin and is generally accompanied by mitral and aortic valve involvement.
[1]

Most stenotic tricuspid valves are associated with clinical evidence of regurgitation that can be documented by performing a physical examination (murmur), echocardiography, or angiography. Stenotic tricuspid valves are always anatomically abnormal, and the cause is limited to a few conditions. With the exceptions of congenital causes or active infective endocarditis, tricuspid stenosis takes years to develop.
[2, 3]

A representation of a stenotic tricuspid valve. This image demonstrates fusion of the commissures (shown as dotted lines).

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Pathophysiology

Tricuspid stenosis results from alterations in the structure of the tricuspid valve that precipitate inadequate excursion of the valve leaflets. The most common etiology is rheumatic fever, and tricuspid valve involvement occurs universally with mitral and aortic valve involvement. With rheumatic tricuspid stenosis, the valve leaflets become thickened and sclerotic as the chordae tendineae become shortened. The restricted valve opening hampers blood flow into the right ventricle and, subsequently, to the pulmonary vasculature. Right atrial enlargement is observed as a consequence. The obstructed venous return results in hepatic enlargement, decreased pulmonary blood flow, and peripheral edema. Other rare causes of tricuspid stenosis include carcinoid syndrome, endocarditis, endomyocardial fibrosis, systemic lupus erythematosus, and congenital tricuspid atresia.
[2, 3, 4]

In the rare instances of congenital tricuspid stenosis, the valve leaflets may manifest various forms of deformity, which can include deformed leaflets, deformed chordae, and displacement of the entire valve apparatus. Other cardiac anomalies are usually present.
[1]

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Epidemiology

United States data

Tricuspid stenosis is rare, occurring in less than 1% of the population. While found in approximately 15% of patients with rheumatic heart disease at autopsy, it is estimated to be clinically significant in only 5% of these patients. The incidence of the congenital form of the disease is less than 1%.

International data

Tricuspid stenosis is found in approximately 3% of the international population. It is more prevalent in areas with a high incidence of rheumatic fever. The congenital form of the disease is rare and true incidence is not available.

Race-, sex-, and age-related demographics

No racial predisposition is apparent.

Tricuspid stenosis is observed more commonly in women than in men, similar to mitral stenosis of rheumatic origin. The congenital form of the disease has a slightly higher male predominance.

Tricuspid stenosis can present as a congenital lesion or later in life when it is due to some other condition. The congenital form accounts for approximately 0.3% of all congenital heart disease cases. The frequency of tricuspid stenosis in the older population, due to secondary causes, ranges from 0.3-3.2%.

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Prognosis

Mortality/Morbidity

The mortality associated with tricuspid stenosis depends on the precipitating cause. The general mortality rate is approximately 5%.

Disclosure: Serve(d) as a speaker or a member of a speakers bureau for: Actelion, Bayer, Gilead, Lung Biotechnology, United Therapeutics<br/>Received research grant from: Actelion, Bayer, Gilead, Ikaria, Lung Biotechnology, Pfizer, Reata, United Therapeutics<br/>Received income in an amount equal to or greater than $250 from: Actelion, Bayer, Gilead, Lung Biotechnology, Medtronic, Reata, United Therapeutics.